The diagnosis of hyperimmunoglobulinemia E (hyper lgE) or Job·s syndrome was made in a live-month-old girl with chronic staphylococcal mastitis. elevated ...
CASE REPORT
Management of recurrent lung infections in a case of hyperimmunoglobulinemia E (or Job's) syndrome M AR SENAULT MD, V E CHAVE MD FRCSC , C F ISH MD FRCPC, J- P P RAUD MD PhD Departments of Pediatrics, Surgery and Radiology, Centre hospitalier universitaire de Sherbrooke, Faculty of Medicine, Universite de Sherbrooke, Sherbrooke, Quebec M ARSENAULT, V ECHAVE, C FISll,J-P PH.AUD. Management of recurrent lung infections in a case of hyperimmunoglobulinemia E (or Job's) syndrome. Can Respir .J 1995;2(1 ):77-80.
The diagnosis of hyperimmunoglobulinemia E (hyper lgE) or Job· s syndrome was made in a li ve-month-old girl with chroni c staphylococcal mastitis. elevated serum IgE and abnorm al neutrophil chemotaxis. Arte r mult ipl e hospitalizations for severe- skin in fcc tions. ri ght upper lobe bu llae were found and treated by lobectomy when the patient was three years of age. Thereafter, the pat ient was repeatedly hospi tal ized for pneumonia whil e on clox aci lli n prophylaxis and rece iv ing regu lar chest physiotherapy. Whe n she was 12 years old. pulmonary detcri orat ion (increased frequency or pulmonary in l'ct.:tions , hemoptysis. rad iological destructi on or the ri ght middle lobe) led 10 a ri gh t middle lobectomy. Si nce thi s intervent ion. the pat ient has had an improved quality nf life, takes part in regu lar sports activities. wit hout recurrence of severe pulmonary in fectio ns. and has had ne,u- normal pulmon;uy funcli on stud ies. A concerted mcd icosurgic,il therapeutic regi men can control severe pulmon.:uy cumplicatinns in patients with this rare syndrome. Key Words: l J.11wri11111111110gloh11/i11 ,'111i11 L srn dnmu ·. Joh·.,· .H11dru111 e. L1111g u h.H't'SS
Prise en charge des infections pulmonaires recurrentes dans un cas d'hyperimmunoglobulinemie E (syndrome de Job) RESUME : Le di agnostic d'hypcrimmunoglobulinem ie E (hyper lgE) ou syndrome de Job a ele pose chez une fill ettc de 5 mo is prcsen tant unc mas tile staphy lococc ique chroni qu e. un taux e lev e d' lg E ser iqu es ct un ch imiotacti smc des neut rophil es anormal. Apres de nombreu ses hospital isati ons pour des infect ions cutanees graves. une bu ll c a ete decelee dans le lobe su pcrieur droi l et rraitee par lobectomie a !'age de trois ans. Par la suite. la patiente a etc hospitali sec plusicu rs fois pour des pneu monies mal gre une prophylax ie i1 la cloxac illi ne ct une physiotherapi e pulmonaire reguli ere. A r age de 12 ans. une deterioration pulmonai re (frequence accrue des infections pulmonaires, hemoplysies, signes radiologiques d'u ne destruction du lobe moyen ) a neccssitc une lobcctomie du lobe moycn. Depuis cc tte in tervention. la pat icnlc a vu sa qu alite de vie s· ameliorer et parlicipe ~. des activites sporlives regulicres sans nouveaux episodes d'infections pulmonaires graves. Ses epreuve. de fonction pulmonaire son! proches cle la normalc. Un regi me thcrapeutiq ue medicoch irurgical concen e peut enrayer lcs L'Omp liL·ations pulmona ires graves cl1e1, les pati ents souffra nt de ce ra re synd rome.
Currespondence and repri11ts: /Jr Jca11-P1111/ J'm11d. Departe111e111 d,- l'fdia tn,-. Farnlre de Medecine. U11~1·ersir,: de Sf,,,,-hrook,-.. WO/. Nort h / 2th A ,·e1111e. Sherbrooke. (}11 f hcc .1111 5N.J. Tcl,·11hn11e (81 '/) 5/i.i-5555. e.rr ./851. Fax (8 /9) 564-52 t.,
Can Respir J Vol 2 No 1 Spring 1995
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ARSENAULT ET AL
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l IE HY PERI MMUNOG LOB LINEM IA E (HY PER. !GE) OR.
Job ·s syndrome is a rare disease with e levated IgE and a Jefect in immu ne function ( l ,2) res ulting in repeate d s inopu lrnonary tract infections in childhood which eve ntually lead to serious pulmonary J estruction a nd respiratory compromise (3.4). Th is report describes a patient wi th hy per IgE syndrome and severe pul monary complications who now has acceptable pulmonary func tion a nd e njoys a good quality of Jile because of a complete medicosurgical therape utic regimen including tailored pulmonary surgery, physiotherapy and bronchodi l::itor the rapy.
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CASE REPORT T he five- month-old patie nt was diagnosed wit h hyper IgE syndrome when she presented to the pediatric clinic with a history of chronic right breast discharge s ince birth. chronic seborrheic J ermatitis. perforated otitis media and le ft cervical aden itis. Family history was pos itive for a maternal uncle with repeated cutaneous abscesses, pneumonia and multiple bouts of otitis media as a child. two maternal aunts with ch ildhood cutaneous abscesses. and several other maternal relati ves w ith chronic severe pustular lesions of the face and scalp. Physical e xamination revealed an active well looking afebrilc child wi th infected facial eczema. normal c hest, cardiovasc ular and abdominal examination. le ft posterior cervical adenitis and a right breast abscess. Laboratory work up revea led an increaseJ scrum IgE level ( 113 U/mL. which later increased to 15,000 U/m L) and decreased chemotaxis (assessed by the Boyden chamber technique 151). LeukoLJ . cyte count was 21.000x IO /L with 8'7r polymorphonuclear cells, 88'7' lymphocytes, l 7a monocytes and 2% eosinophils. Cu lture o r scalp. breast J ischarge and the cervical abscess were positi ve fo r Stap/irfoco< ·cus rwreus. Review of he r chart showed a positive S auffus culture of the umbilicus at two days of age. By the age of three years. the patient had had th ree hospitalizations, the first for right leg and perianal abscesses. the second for conjunctivitis and an infraorbital abscess. a nd the thi rd for severe puru lent conjunctivi tis. All were cultureposit ive fo r S 011re11s. The episodes o,·curred while the patient was on oral clox::icillin prophylaxis and she responded well to a ntistaphylococcal intrave nous an ti biotics. A lso, at 14 months o ld, she was fou nd to have congenital d islocati(lll uf the hip and later an associated genu valgum. which requ ired three hospitalizations for surg i al correct ion. At the age o f th ree years the patient began to develop pulmonary complications. W hile she was hospitaliLcd for a severe ca mpylobacter gastroente ritis. routine rocntge nograms showed large bullac occupying the enti re right lung field. The patient was asymptomat ic and had normal blood gases. Perfusion lung scan showed almost complete loss of perfusion to the ent ire ri ght lung but normal left lung perfus ion. After four weeks of cloxacil lin the pat ient had slight dyspnea on effort. a nd repeat chest roentgenograms showed bul lae originating in the righ t uppe r lobe and compressing the righ t m iddle and lower lobes (Fi0 urc I). Two months later the patie nt underwent ;1 right superinr lobectomy, which was 78
Figure I) Posteroo111erior (top) w1d fureru/ (bottom) ch
